(18)FLUORO-2-DEOXYGLUCOSE ((18)FDG) PET SCAN OF THE BRAIN IN GLUTARICACIDURIA TYPE-1 - CLINICAL AND MRI CORRELATIONS

The clinical, PET (positron emission tomography) and MRI (magnetic res onance imaging) findings of brain studies in eight patients, previousl y diagnosed to have glutaric aciduria type 1, were retrospectively rev iewed. The neurological findings typically consisted of variable degre es of dementia and extrapyramidal symptoms (dystonia, choreoathetosis and rigidity). Both MRI and PET showed involvement of the putamina in all the patients. The PET scan demonstrated lesions in the head of the caudate nuclei in all of the patients. Brain atrophy, and in particul ar the characteristically-enlarged Sylvian fissures, was better demons trated by MRI. On the other hand, the cerebral cortex and thalamic str uctures were found to be normal by MRI in all patients, whereas PET sc an showed decreased uptake in the cerebral cortex in seven, and in the thalami in three patients. Correlation between imaging and clinical f indings was found to be good when both PET scan and MRI findings of th e brain were taken into consideration. Therefore, the functional (PET) and structural (MRI) studies of the brain were complementary in the i maging evaluation of glutaric aciduria type 1. (C) 1998 Elsevier Scien ce B.V. All rights reserved.